GYNECOLOGY AND OBSTETRICS RESEARCH PUBLISHERS ISSN 2377-1542 OpenJournal A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report Article information Received: August 3 rd , 2022; Revised: August 19 th , 2022; Accepted: August 23 rd , 2022; Published: August 26 th , 2022 Cite this article Deneke T,Tefera A, Girma W, Assefa F, Alemu D, Gossaye B. A rare case of vulvar dermatofibrosarcoma protuberans: A case report. Gynecol Obstet Res Open J. 2022; 8(1): 15-18. doi: 10.17140/GOROJ-8-157 Case Report * Corresponding author Tewodros Deneke, MD Assistant Professor, Department of Pathology, Jimma University Medical Center (JUMC), P. O. Box 5058, Jimma, Ethiopia; E-mail: tednew09@gmailcom Tewodros Deneke, MD * ; Abel Tefera, MD; Woubshet Girma, MD; Fanta Assefa, MD; Demsew Alemu, MD; Bahiru Gossaye, MD Jimma University Medical Center (JUMC), P. O. Box 5058, Jimma, Ethiopia Case Report | Volume 8 | Number 1| 15 ABSTRACT Copyright 2022 by Deneke T. This is an open-access article distributed under Creative Commons Attribution 4.0 International License (CC BY 4.0), which allows to copy, redistribute, remix, transform, and reproduce in any medium or format, even commercially, provided the original work is properly cited. cc Background Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin with high local recurrence rate that rarely presents in the vulva. It affects adults between the second and fifth decade of life and the involved areas are most frequently the trunk, proximal extremities, head and neck. Case Presentation A 45-years-old Para 4 women presented with two raised masses on the right part of vulva for the last 17-month duration which in- crease in size for the last 6-month. First, incisional biopsy was taken from the mass and diagnosed as “Suggestive of leiomyoma”. Fol- lowing to this, excision of the whole mass was done and sent for histopathologic examination. The final histopathologic diagnosis became DFSP with the classic microscopic picture of a storiform and honeycomb pattern of monomorphic bland spindle cells. Conclusion Dermatofibrosarcoma protuberans infrequently involves the vulva and should be considered in the differential diagnosis of other spindle cell lesions presenting in this unusual site. Keywords Dermatofibrosarcoma protuberance; Leiomyoma; Vulva; Spindle cell lesions. INTRODUCTION D ermatofibrosarcoma protuberans (DFSP) is a low-to-inter- mediate grade sarcoma of dermal origin that rarely presents in the vulva. 1 It is an uncommon soft tissue tumor that has a high local recurrence rate with an incidence of 0.1% of all cancers and 1% of all soft tissue sarcomas. 2 It affects adults between the sec- ond and fifth decade of life and the involved areas are most fre- quently the trunk, proximal extremities, head and neck. 3 DFSP is flesh-colored or slightly yellow–brown skin tu- mour without epidermal invasion but with intracutaneous and sub- cutaneous spread. Sometimes the tumour presents as a reddish, flat elevated, firm lesion with irregular borders or multinodular appear- ance. 4 Histopathology with immunohistochemical findings is used to diagnose DFSP. 3 As metastasis is rare, morbidity due to local recurrence is a more common issue. 5 The standard treatment techniques for resectable DFSP are complete surgical excision with wide local excisions of 2 or 3 cm tumor-free margins whereas; un- resectable DFSPs are treated with radiation therapy, which improve local control and reduce the risk of recurrence post-operatively. so, early recognition of DFSP is extremely important because of the excellent prognosis following adequate excision. 6-9 Here we report a new case of DFSP on vulvar area which was pre-operatively diagnosed with incisional histopathology to be Leiomyoma. CASE PRESENTATION A 45-years-old Para 4 women from rural area presented in Jimma University Medical Center (JUMC) for complaint of swelling on right vulvar area for the last 17-month duration which increase in size for the last 6-month. She visited local traditional healer 3-month back, ointment given to be applied daily base and started to have discharge with blood from the mass, itching sensation and pain.
A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report
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ISSN 2377-1542
Open Journal
A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report
Article information Received: August 3rd, 2022; Revised: August 19th, 2022; Accepted: August 23rd, 2022; Published: August 26th, 2022
Cite this article Deneke T, Tefera A, Girma W, Assefa F, Alemu D, Gossaye B. A rare case of vulvar dermatofibrosarcoma protuberans: A case report. Gynecol Obstet Res Open J. 2022; 8(1): 15-18. doi: 10.17140/GOROJ-8-157
Case Report
*Corresponding author Tewodros Deneke, MD Assistant Professor, Department of Pathology, Jimma University Medical Center (JUMC), P. O. Box 5058, Jimma, Ethiopia; E-mail: tednew09@gmailcom
Tewodros Deneke, MD*; Abel Tefera, MD; Woubshet Girma, MD; Fanta Assefa, MD; Demsew Alemu, MD; Bahiru Gossaye, MD
Jimma University Medical Center (JUMC), P. O. Box 5058, Jimma, Ethiopia
Case Report | Volume 8 | Number 1| 15
ABSTRACT
Copyright 2022 by Deneke T. This is an open-access article distributed under Creative Commons Attribution 4.0 International License (CC BY 4.0), which allows to copy, redistribute, remix, transform, and reproduce in any medium or format, even commercially, provided the original work is properly cited. cc
Background Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin with high local recurrence rate that rarely presents in the vulva. It affects adults between the second and fifth decade of life and the involved areas are most frequently the trunk, proximal extremities, head and neck. Case Presentation A 45-years-old Para 4 women presented with two raised masses on the right part of vulva for the last 17-month duration which in- crease in size for the last 6-month. First, incisional biopsy was taken from the mass and diagnosed as “Suggestive of leiomyoma”. Fol- lowing to this, excision of the whole mass was done and sent for histopathologic examination. The final histopathologic diagnosis became DFSP with the classic microscopic picture of a storiform and honeycomb pattern of monomorphic bland spindle cells. Conclusion Dermatofibrosarcoma protuberans infrequently involves the vulva and should be considered in the differential diagnosis of other spindle cell lesions presenting in this unusual site.
Keywords Dermatofibrosarcoma protuberance; Leiomyoma; Vulva; Spindle cell lesions.
INTRODUCTION
Dermatofibrosarcoma protuberans (DFSP) is a low-to-inter- mediate grade sarcoma of dermal origin that rarely presents
in the vulva.1 It is an uncommon soft tissue tumor that has a high local recurrence rate with an incidence of 0.1% of all cancers and 1% of all soft tissue sarcomas.2 It affects adults between the sec- ond and fifth decade of life and the involved areas are most fre- quently the trunk, proximal extremities, head and neck.3
DFSP is flesh-colored or slightly yellow–brown skin tu- mour without epidermal invasion but with intracutaneous and sub- cutaneous spread. Sometimes the tumour presents as a reddish, flat elevated, firm lesion with irregular borders or multinodular appear- ance.4
Histopathology with immunohistochemical findings is used to diagnose DFSP.3 As metastasis is rare, morbidity due to local recurrence is a more common issue.5 The standard treatment
techniques for resectable DFSP are complete surgical excision with wide local excisions of 2 or 3 cm tumor-free margins whereas; un- resectable DFSPs are treated with radiation therapy, which improve local control and reduce the risk of recurrence post-operatively. so, early recognition of DFSP is extremely important because of the excellent prognosis following adequate excision.6-9
Here we report a new case of DFSP on vulvar area which was pre-operatively diagnosed with incisional histopathology to be Leiomyoma.
CASE PRESENTATION
A 45-years-old Para 4 women from rural area presented in Jimma University Medical Center (JUMC) for complaint of swelling on right vulvar area for the last 17-month duration which increase in size for the last 6-month. She visited local traditional healer 3-month back, ointment given to be applied daily base and started to have discharge with blood from the mass, itching sensation and pain.
Gynecol Obstet Res Open J. 2022 8(1): 15-18. doi: 10.17140/GOROJ-8-157
16 Case Report | Volume 8 | Number 1|
Her Gynecologic evaluation shows stable vital signs and two raised masses on the right part of vulva. The first one is 10 by 7 cm fleshy, non-tender, round mass with broad based pedicle, with discharge on the surface on the right side of vulva, along the labia majora and the second located on the right upper part of the vulva, non-tender measuring 3×3 cm which extends to subcutaneous tis- sue, but moves separately from underlying structure. The overlying skin of the mass is indurated with hypopigmentation (Figure 1).
Initially Incisional biopsy taken from the mass and di- agnosed as “Suggestive of leiomyoma” but due to rare occurrence of myoma on the vulvar area and difficulty of correlation with clinical presentation of our patient; re-biopsy was taken and the histologic examination showed with intersecting fascicles of bland spindle cells and diagnosed as leiomyoma again (Figure 2).
With the diagnosis of vulvar myoma the patient admitted to Gynecology ward, oncology side and she was put on perineal care BID (two times a day), the mass become clean and excision done 3-days after admission. Intra-operatively the mass with indu- rated skin localized clearly, incision put circumferentially, around both masses together, with 2-3 cm of margin to include indurat- ed skin and there was plane of separation between the mass and underlying tissue, through which removal of the mass completed and sent for pathologic examination. The defect evaluated, 2 cm depth at the center, there was no remaining mass and changed skin, closed in two layers with vicryl no 2/0 in interrupted manner and the skin also closed with the same manner (Figure 3).
Post-operatively the patient kept at bed rest for 72-hours to reduce tension on the surgical site and discharged on 7th post- operative day after she started ambulation fully and no problem with surgical site.
Histological examination of the excised mass demon- strated a storiform and honeycomb pattern composed of Mono- morphic bland fibroblast like spindle cells extending into the sur- rounding fibro-adipose tissue with areas of Myxoid change and all margins were involved. Based on the morphologic pattern, a diag- nosis of dermatofibrosarcoma protuberans with positive margin was rendered. After six-week of the procedure the patient is fine, the surgical site healed and started her regular sexual intercourse (Figure 4).
DISCUSSION
Dermatofibrosarcoma protuberans is an uncommon soft tissue tumor characterized by a low propensity for metastasis and a high rate of local recurrence, it rarely presents in the vulva.10
DFSP usually occurs as a solitary lesion, but can also present with multiple foci.11 The tumor is often solid and due to its indolent nature, often escapes detection in the early stages.12 DFSP has a distinctive histologic appearance but can mimic other diseases. The morphologic and molecular pathological findings of vulvar DFSP are similar to DFSP in other sites.13,14 a variety of di- agnoses, such as cellular dermatofibroma, cellular leiomyoma, neu- rofibroma, low-grade leiomyosarcoma, fibrosarcoma, low-grade
Figure 4. Honeycomb Pattern Infiltrating in to Lobules of Subcutaneous fat (H and E,*100) A and B Uniform Population of Slender Fibroblasts Arranged in Storiform Pattern (H and E,*100 and 400) C and D
A B
Figure 1. Picture Taken after the Mass has been Cleaned, Shows Mass Arise from Indurated Skin
Figure 2. Intersecting Fascicles of Bland Spindle Cells
Figure 3. Intraoperative Picture A Shows a Defect where the Mass Removed B, after the Defect Closed in Layer
Gynecol Obstet Res Open J. 2022 8(1): 15-18. doi: 10.17140/GOROJ-8-157
17Case Report | Volume 8 | Number 1|
malignant schwannoma, desmoplastic melanoma, cellular neuro- fibroma, and low-grade malignant peripheral nerve sheet tumor were initially considered in some tumors later diagnosed as DFSP. This finding underscores the importance of proper recognition of the histologic features diagnostic of DFSP and the use of CD34 immunostaining to confirm this diagnosis.15 Our patient was also diagnosed as leiomyoma initially.
DFSP is characterized by the presence of slender spindle cells arranged in a whorled pattern at the center of the tumor. The superficial portion of the tumor is hypocellular with a mixture of spindle cells and dermal collagen, whereas, in the tumor deep portion, the spindle cells infiltrate the fat creating a honeycomb appearance. Nuclear pleomorphism is absent, and the mitotic in- dex is usually up to 5 mitoses per 10 high power fields (HPFs).16,17 Our patient’s cells demonstrated this classic storiform pattern with monotonous spindle cells and also invaded the subcutaneous fat. Histologically, several variants of DFSP have been described and it is important they are well-characterized to avoid misdiagnosis with other types of tumors. These variants include pigmented, myxoid, myoid, granular cell, sclerotic, atrophic DFSP and giant cell fibro- blastoma.18
Immunohistochemically, DFSP is diffusely and strongly positive for CD34 and vimentin and negative for S-100, smooth muscle actin, desmin, keratin, and epithelial membrane antigen. In addition, it is usually negative for Factor XIII, although scattered cells can be positive for this marker. Translocation of chromo- somes 17 and 22 (t (17:22)) is observed in over 90% of DFSPs.19
To ensure a correct diagnosis, initial biopsy samples must be analyzed using comprehensive immunohistochemical studies, such as CD34- staining, that are capable of distinguishing between DFSP and other spindle-cell tumors.20
The treatment, for DFSP of vulva, is based on surgery, whether primary or recurrent. The principal aim of surgery is to remove the tumor completely, because of the close relationship between residual tumor and local recurrence.21
CONCLUSION
This is a rare case vulvar DFSP that presented with clinical symp- toms of vulvar carcinoma that was initially diagnosed as leio- myoma. Dermatofibrosarcoma protuberans (DFSP) infrequently involves the vulva and should be considered in the differential di- agnosis of other spindle cell lesions presenting in this unusual site.
INSTITUTIONAL REVIEW BOARD
This study has been approved by the Institutional Review Board (IRB).
CONSENT
We would like to thank our patient who consented to participate in this case report.
CONFLICTS OF INTEREST
The authors declare that they have no conflicts of interest.
REFERENCES
1. Gilani S, Al-Khafaji B. Dermatofibrosarcoma protuberans of the vulva: A mesenchymal tumor with a broad differential diagno- sis and review of literature. Pathologica. 2014; 106(4): 338-341.
2. Kontzoglou K, Stamatakos M, Polyzou E, Levidou G, Iannescu R, Safioleas M. Dermatofibrosarcoma: A rare form of soft tissue. Management and review of the literature. Chirurgia (Bucur). 2011; 106(5): 653-656.
3. Chana IL, Carneiroa S, Menezesa M, et al. Dermatofibrosar- coma protuberans: A case report. Case Rep Dermatol. 2014; 6(2): 134-139. doi: 10.1159/000362900
4. Serra-Guillén C, Llombart B, Sanmartín O. Dermatofibrosar- coma protuberans. Actas Dermosifiliogr. 2012; 103: 762-77. doi: 10.1016/j.ad.2011.10.007
5. Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans. FL, USA: StatPearls Publishing; Updated January 21, 2022.
6. Doufekas K, Duncan TJ, Williamson KM, Varma S, Nunns D. Mohs micrographic surgery for dermatofibrosarcoma pro- tuberans of the vulva. Obstet Gynecol Int. 2009; 2009: 1-2. doi: 10.1155/2009/547672
7. Farma JM, Ammori JB, Zager JS, et al. Dermatofibrosarcoma protuberans: how wide should we resect? Ann Surg Oncol. 2010; 17: 2112-2118. doi: 10.1245/s10434-010-1046-8
8. Häfner HM, Moehrle M, Eder S, Trilling B, Röcken M, Breun- inger H. 3DHistological evaluation of surgery in dermatofibro- sarcoma protuberans and malignant fibrous histiocytoma: Differ- ences in growth patterns and outcome. Eur J Surg Oncol. 2008; 34: 680-686. doi: 10.1016/j.ejso.2007.07.004
9. Asuquo ME, Umoh MS, Ebughe G. Dermatofibrosarcoma protuberance: Case reports. Ann Afr Med. 2007; 6(2): 80-83. doi: 10.4103/1596-3519.55710
10. Sun C, Zou J, Wang Q, et al. Review of the pathophysiol- ogy, diagnosis, and therapy of vulvar leiomyoma, a rare gy- necological tumor. J Int Med Res. 2018; 46(2): 663-674. doi: 10.1177/0300060517721796
11. Ozmen E, Güney G, Algin O. Magnetic resonance imaging of vulvar dermatofibrosarcoma protuberans - report of a case. Radiol Oncol. 2013; 47(3): 244-246 . doi: 10.2478/raon-2013-0039
12. Doufekas K, Duncan TJ, Williamson KM, Varma S, Nunns D. Mohs micrographic surgery for dermatofibrosarcoma protu- berans of the vulva. Obstet Gynecol Int. 2009; 2009: 547672. doi: 10.1155/2009/547672
Gynecol Obstet Res Open J. 2022 8(1): 15-18. doi: 10.17140/GOROJ-8-157
18 Case Report | Volume 8 | Number 1|
13. Gokden N, Dehner LP, Zhu X, Pfeifer JD. Dermatofibrosar- coma protuberans of the vulva and groin: Detection of COL1A1- PDGFB fusion transcripts by RT-PCR. J Cutan Pathol. 2003; 30: 190-195. doi: 10.1034/j.1600-0560.2003.00037.x
14. Mbonde MP, Amir H, Kitinya JN. Dermatofibrosarcoma pro- tuberans: A clinicopathological study in an African population. East Afr Med J. 1996; 73: 410-413.
15. Edelweiss M, Malpica A. Dermatofibrosarcoma protuberans of the vulva: A clinicopathologic and immunohistochemical study of 13 cases. Am J Surg Pathol. 2010; 34(3): 393-400. doi: 10.1097/ PAS.0b013e3181cf7fc1
16. Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS. Incidence and survival of primary dermatofibro- sarcoma protuberans in the United States. Dermatol Surg. 2016; 42 Suppl 1: S24-S31. doi: 10.1097/DSS.0000000000000300
17. Weiss SW, Goldblum JR. Benign fibrohistiocytic tumors. In: Enzinger FM, Weiss SW, eds. Enzinger & Weiss’s Soft Tissue Tumors.
Missouri, USA: Mosby, Elsevier, Inc; 2008.
18. Lyu A, Wang Q. Dermatofibrosarcoma protuberans: A clini- cal analysis. Oncol Lett. 2018; 16(2): 1855-1862. doi: 10.3892/ ol.2018.8802
19. Weyers W, Mentzel T, Kasper RC, et al. Soft tissue tumours: Fi- brous, fibrohistiocytic and histocytic tumours. In: LeBoit PE BG, Weedon D, Sarasain A, eds. World Health Organization Classification of Tumours Pathology and Genetics of Skin Tumours. Lyon, France: IARC Press; 2006: 254-262.
20. Llombart B, Serra-Guillén C, Monteagudo C, López Guerrero JA, Sanmartin O. Dermatofibrosarcoma protuberans: A compre- hensive review and update on diagnosis and management. SeminDi- agn Patho. 2013; 30: 13-28. doi: 10.1053/j.semdp.2012.01.002
21. Wang Y, Wang Y, Chen R, Tang Z, Liu S. A rare malignant dis- ease, dermatofibrosarcoma protuberans of the breast: A retrospec- tive analysis and review of literature. Biomed Res Int. 2020; 2020: 8852182. doi: 10.1155/2020/8852182
Submit your article to this journal | https://openventio.org/submit-manuscript/
Open Journal
A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report
Article information Received: August 3rd, 2022; Revised: August 19th, 2022; Accepted: August 23rd, 2022; Published: August 26th, 2022
Cite this article Deneke T, Tefera A, Girma W, Assefa F, Alemu D, Gossaye B. A rare case of vulvar dermatofibrosarcoma protuberans: A case report. Gynecol Obstet Res Open J. 2022; 8(1): 15-18. doi: 10.17140/GOROJ-8-157
Case Report
*Corresponding author Tewodros Deneke, MD Assistant Professor, Department of Pathology, Jimma University Medical Center (JUMC), P. O. Box 5058, Jimma, Ethiopia; E-mail: tednew09@gmailcom
Tewodros Deneke, MD*; Abel Tefera, MD; Woubshet Girma, MD; Fanta Assefa, MD; Demsew Alemu, MD; Bahiru Gossaye, MD
Jimma University Medical Center (JUMC), P. O. Box 5058, Jimma, Ethiopia
Case Report | Volume 8 | Number 1| 15
ABSTRACT
Copyright 2022 by Deneke T. This is an open-access article distributed under Creative Commons Attribution 4.0 International License (CC BY 4.0), which allows to copy, redistribute, remix, transform, and reproduce in any medium or format, even commercially, provided the original work is properly cited. cc
Background Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin with high local recurrence rate that rarely presents in the vulva. It affects adults between the second and fifth decade of life and the involved areas are most frequently the trunk, proximal extremities, head and neck. Case Presentation A 45-years-old Para 4 women presented with two raised masses on the right part of vulva for the last 17-month duration which in- crease in size for the last 6-month. First, incisional biopsy was taken from the mass and diagnosed as “Suggestive of leiomyoma”. Fol- lowing to this, excision of the whole mass was done and sent for histopathologic examination. The final histopathologic diagnosis became DFSP with the classic microscopic picture of a storiform and honeycomb pattern of monomorphic bland spindle cells. Conclusion Dermatofibrosarcoma protuberans infrequently involves the vulva and should be considered in the differential diagnosis of other spindle cell lesions presenting in this unusual site.
Keywords Dermatofibrosarcoma protuberance; Leiomyoma; Vulva; Spindle cell lesions.
INTRODUCTION
Dermatofibrosarcoma protuberans (DFSP) is a low-to-inter- mediate grade sarcoma of dermal origin that rarely presents
in the vulva.1 It is an uncommon soft tissue tumor that has a high local recurrence rate with an incidence of 0.1% of all cancers and 1% of all soft tissue sarcomas.2 It affects adults between the sec- ond and fifth decade of life and the involved areas are most fre- quently the trunk, proximal extremities, head and neck.3
DFSP is flesh-colored or slightly yellow–brown skin tu- mour without epidermal invasion but with intracutaneous and sub- cutaneous spread. Sometimes the tumour presents as a reddish, flat elevated, firm lesion with irregular borders or multinodular appear- ance.4
Histopathology with immunohistochemical findings is used to diagnose DFSP.3 As metastasis is rare, morbidity due to local recurrence is a more common issue.5 The standard treatment
techniques for resectable DFSP are complete surgical excision with wide local excisions of 2 or 3 cm tumor-free margins whereas; un- resectable DFSPs are treated with radiation therapy, which improve local control and reduce the risk of recurrence post-operatively. so, early recognition of DFSP is extremely important because of the excellent prognosis following adequate excision.6-9
Here we report a new case of DFSP on vulvar area which was pre-operatively diagnosed with incisional histopathology to be Leiomyoma.
CASE PRESENTATION
A 45-years-old Para 4 women from rural area presented in Jimma University Medical Center (JUMC) for complaint of swelling on right vulvar area for the last 17-month duration which increase in size for the last 6-month. She visited local traditional healer 3-month back, ointment given to be applied daily base and started to have discharge with blood from the mass, itching sensation and pain.
Gynecol Obstet Res Open J. 2022 8(1): 15-18. doi: 10.17140/GOROJ-8-157
16 Case Report | Volume 8 | Number 1|
Her Gynecologic evaluation shows stable vital signs and two raised masses on the right part of vulva. The first one is 10 by 7 cm fleshy, non-tender, round mass with broad based pedicle, with discharge on the surface on the right side of vulva, along the labia majora and the second located on the right upper part of the vulva, non-tender measuring 3×3 cm which extends to subcutaneous tis- sue, but moves separately from underlying structure. The overlying skin of the mass is indurated with hypopigmentation (Figure 1).
Initially Incisional biopsy taken from the mass and di- agnosed as “Suggestive of leiomyoma” but due to rare occurrence of myoma on the vulvar area and difficulty of correlation with clinical presentation of our patient; re-biopsy was taken and the histologic examination showed with intersecting fascicles of bland spindle cells and diagnosed as leiomyoma again (Figure 2).
With the diagnosis of vulvar myoma the patient admitted to Gynecology ward, oncology side and she was put on perineal care BID (two times a day), the mass become clean and excision done 3-days after admission. Intra-operatively the mass with indu- rated skin localized clearly, incision put circumferentially, around both masses together, with 2-3 cm of margin to include indurat- ed skin and there was plane of separation between the mass and underlying tissue, through which removal of the mass completed and sent for pathologic examination. The defect evaluated, 2 cm depth at the center, there was no remaining mass and changed skin, closed in two layers with vicryl no 2/0 in interrupted manner and the skin also closed with the same manner (Figure 3).
Post-operatively the patient kept at bed rest for 72-hours to reduce tension on the surgical site and discharged on 7th post- operative day after she started ambulation fully and no problem with surgical site.
Histological examination of the excised mass demon- strated a storiform and honeycomb pattern composed of Mono- morphic bland fibroblast like spindle cells extending into the sur- rounding fibro-adipose tissue with areas of Myxoid change and all margins were involved. Based on the morphologic pattern, a diag- nosis of dermatofibrosarcoma protuberans with positive margin was rendered. After six-week of the procedure the patient is fine, the surgical site healed and started her regular sexual intercourse (Figure 4).
DISCUSSION
Dermatofibrosarcoma protuberans is an uncommon soft tissue tumor characterized by a low propensity for metastasis and a high rate of local recurrence, it rarely presents in the vulva.10
DFSP usually occurs as a solitary lesion, but can also present with multiple foci.11 The tumor is often solid and due to its indolent nature, often escapes detection in the early stages.12 DFSP has a distinctive histologic appearance but can mimic other diseases. The morphologic and molecular pathological findings of vulvar DFSP are similar to DFSP in other sites.13,14 a variety of di- agnoses, such as cellular dermatofibroma, cellular leiomyoma, neu- rofibroma, low-grade leiomyosarcoma, fibrosarcoma, low-grade
Figure 4. Honeycomb Pattern Infiltrating in to Lobules of Subcutaneous fat (H and E,*100) A and B Uniform Population of Slender Fibroblasts Arranged in Storiform Pattern (H and E,*100 and 400) C and D
A B
Figure 1. Picture Taken after the Mass has been Cleaned, Shows Mass Arise from Indurated Skin
Figure 2. Intersecting Fascicles of Bland Spindle Cells
Figure 3. Intraoperative Picture A Shows a Defect where the Mass Removed B, after the Defect Closed in Layer
Gynecol Obstet Res Open J. 2022 8(1): 15-18. doi: 10.17140/GOROJ-8-157
17Case Report | Volume 8 | Number 1|
malignant schwannoma, desmoplastic melanoma, cellular neuro- fibroma, and low-grade malignant peripheral nerve sheet tumor were initially considered in some tumors later diagnosed as DFSP. This finding underscores the importance of proper recognition of the histologic features diagnostic of DFSP and the use of CD34 immunostaining to confirm this diagnosis.15 Our patient was also diagnosed as leiomyoma initially.
DFSP is characterized by the presence of slender spindle cells arranged in a whorled pattern at the center of the tumor. The superficial portion of the tumor is hypocellular with a mixture of spindle cells and dermal collagen, whereas, in the tumor deep portion, the spindle cells infiltrate the fat creating a honeycomb appearance. Nuclear pleomorphism is absent, and the mitotic in- dex is usually up to 5 mitoses per 10 high power fields (HPFs).16,17 Our patient’s cells demonstrated this classic storiform pattern with monotonous spindle cells and also invaded the subcutaneous fat. Histologically, several variants of DFSP have been described and it is important they are well-characterized to avoid misdiagnosis with other types of tumors. These variants include pigmented, myxoid, myoid, granular cell, sclerotic, atrophic DFSP and giant cell fibro- blastoma.18
Immunohistochemically, DFSP is diffusely and strongly positive for CD34 and vimentin and negative for S-100, smooth muscle actin, desmin, keratin, and epithelial membrane antigen. In addition, it is usually negative for Factor XIII, although scattered cells can be positive for this marker. Translocation of chromo- somes 17 and 22 (t (17:22)) is observed in over 90% of DFSPs.19
To ensure a correct diagnosis, initial biopsy samples must be analyzed using comprehensive immunohistochemical studies, such as CD34- staining, that are capable of distinguishing between DFSP and other spindle-cell tumors.20
The treatment, for DFSP of vulva, is based on surgery, whether primary or recurrent. The principal aim of surgery is to remove the tumor completely, because of the close relationship between residual tumor and local recurrence.21
CONCLUSION
This is a rare case vulvar DFSP that presented with clinical symp- toms of vulvar carcinoma that was initially diagnosed as leio- myoma. Dermatofibrosarcoma protuberans (DFSP) infrequently involves the vulva and should be considered in the differential di- agnosis of other spindle cell lesions presenting in this unusual site.
INSTITUTIONAL REVIEW BOARD
This study has been approved by the Institutional Review Board (IRB).
CONSENT
We would like to thank our patient who consented to participate in this case report.
CONFLICTS OF INTEREST
The authors declare that they have no conflicts of interest.
REFERENCES
1. Gilani S, Al-Khafaji B. Dermatofibrosarcoma protuberans of the vulva: A mesenchymal tumor with a broad differential diagno- sis and review of literature. Pathologica. 2014; 106(4): 338-341.
2. Kontzoglou K, Stamatakos M, Polyzou E, Levidou G, Iannescu R, Safioleas M. Dermatofibrosarcoma: A rare form of soft tissue. Management and review of the literature. Chirurgia (Bucur). 2011; 106(5): 653-656.
3. Chana IL, Carneiroa S, Menezesa M, et al. Dermatofibrosar- coma protuberans: A case report. Case Rep Dermatol. 2014; 6(2): 134-139. doi: 10.1159/000362900
4. Serra-Guillén C, Llombart B, Sanmartín O. Dermatofibrosar- coma protuberans. Actas Dermosifiliogr. 2012; 103: 762-77. doi: 10.1016/j.ad.2011.10.007
5. Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans. FL, USA: StatPearls Publishing; Updated January 21, 2022.
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